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一名艾滋病患者合并噬血细胞性淋巴组织细胞增生症的皮下脂膜炎样T细胞淋巴瘤病例

A Case of Subcutaneous Panniculitis-Like T-cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an HIV Patient.

作者信息

Kanitthamniyom Chanakarn, Osorio Alejandra, Saowapa Sakditad, Siladech Pharit

机构信息

Hematology, Thainakarin Hospital, Bangkok, THA.

Internal Medicine, University of Alabama at Birmingham (UAB) Hospital, Birmingham, USA.

出版信息

Cureus. 2023 Nov 28;15(11):e49564. doi: 10.7759/cureus.49564. eCollection 2023 Nov.

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs with hemophagocytic lymphohistocytosis, a life-threatening hyperinflammatory syndrome. The majority of SPTL cases express αβ T-cell receptors (SPTL-AB) and have a favorable prognosis with oral immunosuppressive agents. We report a 37-year-old male patient with HIV infection who had a history of low-grade fever for one year, multiple tender subcutaneous nodules on both thighs, and cytopenia. He received several courses of antibiotics without significant improvement. A random skin biopsy showed lobular panniculitis and he was treated with steroids, but his fever recurred after steroid withdrawal. A second skin biopsy confirmed the diagnosis of SPTL. A bone marrow examination revealed hemophagocytic lymphohistiocytosis. He was successfully treated with cyclosporin A and prednisolone and achieved a complete response after one year of drug discontinuation. Panniculitis-like skin lesions have various etiologies and may present as a clinical mimic of lupus erythematosus panniculitis. The selection of an optimal site for skin biopsy is crucial to avoid erroneous diagnoses and adverse outcomes. We report a case of SPTL in an HIV-positive patient, which illustrates this diagnostic challenge.

摘要

皮下脂膜炎样T细胞淋巴瘤(SPTL)是一种罕见的非霍奇金淋巴瘤亚型,表现为脂膜炎样皮肤病变。它常与噬血细胞性淋巴组织细胞增生症同时出现,这是一种危及生命的高炎症综合征。大多数SPTL病例表达αβ T细胞受体(SPTL-AB),口服免疫抑制剂治疗预后良好。我们报告一例37岁男性HIV感染患者,有一年低热病史,双侧大腿有多个压痛性皮下结节,伴有血细胞减少。他接受了几个疗程的抗生素治疗,无明显改善。随机皮肤活检显示小叶性脂膜炎,给予类固醇治疗,但停用类固醇后发热复发。第二次皮肤活检确诊为SPTL。骨髓检查显示噬血细胞性淋巴组织细胞增生症。他接受环孢素A和泼尼松龙成功治疗,停药一年后达到完全缓解。脂膜炎样皮肤病变有多种病因,可能表现为狼疮性脂膜炎的临床模拟。选择最佳的皮肤活检部位对于避免错误诊断和不良后果至关重要。我们报告一例HIV阳性患者的SPTL病例,说明了这一诊断挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/41b9/10754025/4e5a93397a93/cureus-0015-00000049564-i01.jpg

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